Nephritic Syndrome

Question 1

What is nephritic syndrome?

Answer 1

Glomerular disease characterized by inflammation in the glomeruli and bleeding.

Question 2

What are the common clinical findings in nephritic syndrome?

Answer 2

Azotemia and oliguria
RBC casts and dysmorphic RBCs in the urine
Hypercellular inflammed glomeruli on biopsy

Question 3

What is the nephritic syndrome that arises after group A beta-hemolytic streptoccal infections?

Answer 3

Poststreptococcal Glomerulonephritis

Question 4

What are the symptoms associated with PSGN?

Answer 4

• presents 2-3 weeks after infection.
• cola colored urine (hematuria).
• malaise, fever, nausea
• periorbital edema

Question 5

What is the characteristic finding in PSGN on EM and IF?

Answer 5

EM-> Subepithelial humps

IM-> granular deposits of IgG and C3 in GBM and mesangium

Question 6

What would IF show in PSGN?

Answer 6

Granular immune complex deposition.

Question 7

What two diseases will show a granular immunofluorescence pattern?

Answer 7

1) PSGN

2) Diffuse proliferative Glomerulonephritis

Question 8

What is the name of the nephritic syndrome in which progression to renal failure occurs in weeks to months?

Answer 8

Rapidly progressive Glomerulonephritis

Question 9

What are the characteristic findings in rapidly progressive Glomerulonephritis?

Answer 9

Formation of cresents in the glomeruli.

Question 10

What type of RPGN is characterized by linear deposits of IgG on immunofluorescence?

Answer 10

Goodpasture syndrome. Ab against collagen will be present in the glomeruli and alveolar basement membranes.

Question 11

What types of RPGN are characterized by granular deposits on immunofluorescence?

Answer 11

PSGN and diffuse proliferative glomerulonephritis

Question 12

What type of RPGN is characterized by negative findings on immunofluorescence?

Answer 12

Wegeners granulomatosis–> c-ANCA

Microscopic polyangiitis and Churg-Strauss–> p-ANCA

Question 13

What are the contents of the crescents that are seen in RPGN?

Answer 13

Crescents: proliferation of parietal cells, macrophages, PMNs, and fibrin strands between cells.

• all contained within bowmans space.

Question 14

What is the most common nephropathy worldwide?

Answer 14

IgA nephropathy

Question 15

Where are IgA deposits found in IgA nephropathy?

Answer 15

IgA deposits are found in the mesangium and are detected by immunofluorescence

Question 16

What is the common presentation seen in IgA nephropathy?

Answer 16

• Commonly presents during childhood as episodic or microscopic hematuria with RBC casts, usually following mucosal infections.
• infection causes increase in IgA production.

Question 17

What is the name of the disease that is associated with an inherited defect in type IV collagen and what is its inheritance patten?

Answer 17

Alport syndrome.

X-Linked.

Question 18

What is the result of defective type IV collagen in Alports syndrome?

Answer 18

-Thinning and splitting of the glomerular basement membrane resulting in a defective basement membrane.

Question 19

What are the findings on EM in Alports syndrome?

Answer 19

Alternating thickening and thinning of the glomerular basement membrane.

Question 20

What are the findings on LM, IF, and EM in IgA nephropathy?

Answer 20

LM-> focal mesangial proliferative glomerulonephritis; mesangial widening.

IF-> IgA and C3 in mesangium (can have IgM and IgG present).

EM-> mesangial and paramesangial dense deposits.

Question 21

What is a frequent cause of recurrent gross or microscopic hematuria?

Answer 21

IgA nephropathy

Question 22

What ar ether two eponymic diseases associated with IgA nephropathy?

Answer 22

1) Berger disease-> renal IgA nephropathy-no systemic diseasaae
2) Henoch-schonlein purpura (HSP)-> IgA nephropathy associated with systemic disease, often exhibiting skin (purpuric) manifestations.

Question 23

What age group is primarily affected by IgA nephropathy?

Answer 23

Primarily a disease of older children and young adults ,most common in the 2-3 decades of life.

More common in white poeple and Asians

Men 2:1 women

Question 24

What is a common antecedent infection that can cause a flare up of IgA nephropathy symptoms?

Answer 24

Commonly follows mucosal infections